bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2018–11–18
twenty-one papers selected by
Richard James, University of Pennsylvania
  1. Echocardiography in Congenital Heart Disease.
  2. Birth Location of Infants with Critical Congenital Heart Disease in California.
  3. A case series of different anesthesia approaches for single ventricular physiology patients in various stages of palliation underwent noncardiac procedures.
  4. Early fetal echocardiography.
  5. Prediction of One-Year Transplant-Free Survival after Norwood Procedure Based on the Pre-Operative Data.
  6. Single-center experience of hemodialysis in patients after Fontan palliation.
  7. Effects of Hepatitis C Virus Antibody-Positivity on Cardiac Function and Long-Term Prognosis in Patients With Adult Congenital Heart Disease.
  8. Effect of Congenital Heart Disease Status on Trends in Pediatric Infective Endocarditis Hospitalizations in the United States Between 2000 and 2012.
  9. Use of test weights for breastfeeding infants with congenital heart disease in a cardiac transitional care unit: a best practice implementation project.
  10. Analysis of achieving an "ideal" outcome following midline unifocalization.
  11. Resuscitation for out-of-hospital cardiac arrest in adults with congenital heart disease.
  12. Long-lasting benefits of exercise for those living with a Fontan circulation.
  13. State of the Art Management of Mechanical Heart Valves During Pregnancy.
  14. Extracorporeal Cardiopulmonary Resuscitation: One-Year Survival and Neurobehavioral Outcome Among Infants and Children With In-Hospital Cardiac Arrest.
  15. A Novel Brain Injury Biomarker Correlates with Cyanosis in Infants with Congenital Heart Disease.
  16. Twin pregnancy complicated by total placenta previa in a Fontan-palliated patient: A case report.
  17. Perioperative Exposure to Suspect Neurotoxicants from Medical Devices in Newborns with CHD (Commentary).
  18. Strength training in congenital heart disease: A way to boost respiratory function?
  19. Pediatric Infective Endocarditis and Stroke:A 13-Year Single-Center Review.
  20. Hospital Distribution and Patient Travel Patterns for Congenital Heart Surgery in the United States (Commentary).
  21. Selective serotonin reuptake inhibitor use during early pregnancy and congenital malformations: a systematic review and meta-analysis of cohort studies of more than 9 million births.
  1. Prog Cardiovasc Dis. 2018 Nov 13. pii: S0033-0620(18)30224-X. [Epub ahead of print]
    Echocardiography in Congenital Heart Disease.
    George Mcleod, Kelly Shum, Tripti Gupta, Shourjo Chakravorty, Sergey Kachur, Lisa Bienvenu, Michael White, Sangeeta B Shah.
      As the prevalence of adult congenital heart disease continues to grow secondary to advances in surgical and diagnostic techniques, it is important for a physician to supplement their examinations with non-invasive imaging techniques to assess their patients. Although a number of these patients have regular cardiology followup, some may be new patients that do not even know their cardiac history. Echocardiography has proven to be a useful tool for this purpose and its utility has expanded drastically with the development of better technology and newer techniques. In this article, we highlight some of these advancements including 2D echocardiography, agitated saline, contrast echocardiography, stress, and 4D, in addition to how each modality can help assess key aspects of the structure and function of a congenital heart defect.
    Keywords:  ACHD; Echocardiography; adult congenital heart disease; tetralogy of fallot; transposition of the great vessels
    DOI:  https://doi.org/10.1016/j.pcad.2018.11.004
  2. Pediatr Cardiol. 2018 Nov 10.
    Birth Location of Infants with Critical Congenital Heart Disease in California.
    Neha J Purkey, David M Axelrod, Doff B McElhinney, Joseph Rigdon, FeiFei Qin, Manisha Desai, Andrew Y Shin, Valerie Y Chock, Henry C Lee.
      The American Academy of Pediatrics classifies neonatal intensive care units (NICUs) from level I to IV based on the acuity of care each unit can provide. Birth in a higher level center is associated with lower morbidity and mortality in high-risk populations. Congenital heart disease accounts for 25-50% of infant mortality related to birth defects in the U.S., but recent data are lacking on where infants with critical congenital heart disease (CCHD) are born. We used a linked dataset from the Office of Statewide Health Planning and Development to access ICD-9 diagnosis codes for all infants born in California from 2008 to 2012. We compared infants with CCHD to the general population, identified where infants with CCHD were born based on NICU level of care, and predicted level IV birth among infants with CCHD using logistic regression techniques. From 2008 to 2012, 6325 infants with CCHD were born in California, with 23.7% of infants with CCHD born at a level IV NICU compared to 8.4% of the general population. Level IV birth for infants with CCHD was associated with lower gestational age, higher maternal age and education, the presence of other congenital anomalies, and the diagnosis of a single ventricle lesion. More infants with CCHD are born in a level IV NICU compared to the general population. Future studies are needed to determine if birth in a lower level of care center impacts outcomes for infants with CCHD.
    Keywords:  Birth location; California; Congenital heart disease; Neonatal intensive care; Prevalence
    DOI:  https://doi.org/10.1007/s00246-018-2019-0
  3. Saudi J Anaesth. 2018 Oct-Dec;12(4):12(4): 629-633
    A case series of different anesthesia approaches for single ventricular physiology patients in various stages of palliation underwent noncardiac procedures.
    Abdulaleem Alatassi, Silvia Fernández Mulero, Nancy Massoud, Zainab Alzayer, Ahmed Haroun Mahmoud.
      Patients with single ventricle physiology (SVP) are a particularly challenging population with congenital heart disease (CHD); they will go for staged, palliation ending in the Fontan circulation. Nowadays, with improvement in surgical procedures for CHD, these patients become growing population, and noncardiac surgeries become not uncommon. The authors report different anesthesia approaches for four pediatric patients with SVP underwent ten noncardiac procedures done under general anesthesia following the different stages of palliation at King Abdulaziz Medical City, Riyadh, Kingdom of Saudi Arabia, between 2009 and 2015 and do a brief review of the literature on this topic. The aim of this study is to highlight that anesthesia approach for patients with SVP varies according to the patient physical situation at the time of the procedure, stage of palliation, and type of surgery. Therefore, every anesthesiologist should have thorough knowledge about SVP, different stages of palliative surgery, anesthesia concern in each one and risk factors associated with perioperative morbidity before anesthetizing patients for a noncardiac procedure to keep patient safety as well as avoiding unnecessary cancellation, rescheduling, and admissions to the ward or the Intensive Care Unit.
    Keywords:  Anesthesia of noncardiac surgery; Fontan procedure; Glenn circulation; congenital heart disease; intraoperative complications; postoperative complications; risk factors; single ventricle physiology
    DOI:  https://doi.org/10.4103/sja.SJA_36_18
  4. Birth Defects Res. 2018 Nov 14.
    Early fetal echocardiography.
    Angela McBrien, Lisa K Hornberger.
       OBJECTIVES: To explore the technical aspects and clinical utility of early fetal echocardiography and screening of the fetal heart in early pregnancy. Also, to document differences in cardiac structure and function which can be demonstrated in the late first/early second trimesters.
    METHODS: In addition to summarizing our own experiences of late first/early second trimester fetal echocardiography, we reviewed the literature to explore clinical indications for, technical aspects, safety, accuracy, strengths and weaknesses of early fetal echocardiography.
    RESULTS: In the current era, an increasing number of fetuses are identified as being at risk of congenital heart disease from as early as the late first trimester. In experienced hands, early fetal echocardiography can pick up a high proportion of congenital heart disease with good reliability for the majority of lesions. Early fetal echocardiography is relatively poor at assessing pulmonary veins, the atrioventricular valves and lesions that typically occur later or progress during the course of pregnancy. There is increasing interest in widespread implementation of an early obstetric anomaly screen which includes an assessment of the fetal heart for all pregnancies. There are a variety of hemodynamic differences in the late first/early second trimester compared with later in pregnancy.
    CONCLUSION: Early fetal echocardiography is has become an established tool for detection of congenital heart disease. It affords opportunities to learn about the true spectrum and progression of congenital heart disease in-utero. Operators should be mindful of safety issues, technical aspects and hemodynamic findings which differ when performing echocardiograms at this stage of pregnancy.
    Keywords:  congenital heart disease; fetal echocardiography; first trimester
    DOI:  https://doi.org/10.1002/bdr2.1414
  5. Conf Proc IEEE Eng Med Biol Soc. 2018 Jul;2018 3995-3998
    Prediction of One-Year Transplant-Free Survival after Norwood Procedure Based on the Pre-Operative Data.
    M Luis Ahumadal, Jacquelin Peck, Jorge Guerra, Nhue Do, Monesha Gupta, Sharon Ghazarian, Mohamed Rehman, P Jeffrey Jacobs, And Ali Jalali.
      This paper discusses computational modeling of predictive risk factors for neonates undergoing a Norwood surgical procedure, a multi-stage cardiac procedure that restores functional systemic circulation in patients such as neonates with Hypoplastic Left Heart Syndrome (HLHS). In this model, we apply machine learning based binary classication to 549 cases reported by the Pediatric Heart Networks Single Ventricle Reconstruction Trial. We use neural networks classier to predict risk factors for individual patients undergoing a Norwood procedure for the repair of HLHS. Results indicate that independent risk can be calculated with 85% accuracy and 0.94 area under the receiver operating characteristics curve. This model may help physicians provide counseling for families and medically optimize patients prior to surgery by modifying individual risk factors.
    DOI:  https://doi.org/10.1109/EMBC.2018.8513336
  6. Int J Cardiol Heart Vasc. 2018 Dec;21 94-95
    Single-center experience of hemodialysis in patients after Fontan palliation.
    Brian Young, Wayne Franklin, Wilson Lam, Peter Ermis.
      
    DOI:  https://doi.org/10.1016/j.ijcha.2018.10.008
  7. Am J Cardiol. 2018 Dec 01. pii: S0002-9149(18)31744-2. [Epub ahead of print]122(11): 1965-1971
    Effects of Hepatitis C Virus Antibody-Positivity on Cardiac Function and Long-Term Prognosis in Patients With Adult Congenital Heart Disease.
    Ryo Konno, Shunsuke Tatebe, Koichiro Sugimura, Kimio Satoh, Tatsuo Aoki, Masanobu Miura, Saori Yamamoto, Haruka Sato, Katsuya Kozu, Yosuke Terui, Satoshi Miyata, Osamu Adachi, Masato Kimura, Yoshikatsu Saiki, Hiroaki Shimokawa.
      It was reported that hepatitis C virus (HCV) antibody-positivity adversely affects cardiac function. As the screening for HCV began in 1992, we hypothesized that HCV antibody-positive rate would be high in adult congenital heart disease (ACHD) patients who underwent heart surgery before 1992 and adversely affected cardiac function and long-term prognosis. We retrospectively enrolled 243 ACHD patients (mean age 25.9 years) who underwent cardiac surgery before 1992 and visited our hospital from 1995 to 2015. We compared clinical characteristics including cardiac function and long-term prognosis between HCV antibody-positive (n = 48) and antibody-negative (n = 195) patients. The composite end point (CEP) included cardiac death, heart failure hospitalization, lethal ventricular arrhythmias, and cardiac reoperation. The prevalence of reduced systemic ventricular ejection fraction <50% was significantly higher in the HCV antibody-positive group compared with the HCV antibody-negative group (17 vs 5.4%, p = 0.014). During a mean follow-up period of 10.1 years (interquartile range 6 to 14 years), the CEP was noted in 51 patients. Kaplan-Meier analysis showed the HCV antibody-positive group had significantly poor event-free survival than the HCV antibody-negative group (log-rank, p = 0.002). In contrast, HCV ribonucleic acid-positivity was not a significant predictor of the CEP in the HCV antibody-positive group (log-rank, p = 0.442). Furthermore, the HCV antibody-positivity was significantly associated with the CEP in both univariable and multivariable Cox regression models (hazard ratio 2.37, 95% confident interval 1.32 to 4.15, p = 0.005 and 1.96, 1.06 to 3.63, p = 0.032, respectively). In conclusion, these results suggest that more attention should be paid to HCV antibody-positivity in the management of ACHD patients.
    DOI:  https://doi.org/10.1016/j.amjcard.2018.08.045
  8. Pediatr Cardiol. 2018 Nov 10.
    Effect of Congenital Heart Disease Status on Trends in Pediatric Infective Endocarditis Hospitalizations in the United States Between 2000 and 2012.
    Stephen J Dolgner, Bhawna Arya, Matthew P Kronman, Titus Chan.
      The purpose of this study was to create national estimates for the incidence of pediatric infective endocarditis (IE) in the United States and to determine if these changed after the implementation of the 2007 American Heart Association IE guidelines. It also sought to determine the effect of congenital heart disease (CHD) status on outcomes in IE. Hospital discharges with the diagnosis of IE in patients < 18 years old from the Kids' Inpatient Database were identified from the years 2000, 2003, 2006, 2009, and 2012. Discharges were grouped into Pre- and Post-2007 groups to facilitate analysis surrounding the implementation of the guidelines in 2007. Patients were categorized by age, underlying CHD, and etiologic organism. Descriptive comparisons and changes in categorical variables were made between groups. Average annual IE hospitalization rates before and after the 2007 guidelines were 10.8 and 9.3 per 1,000,000 children, respectively. The proportion of IE patients with CHD was stable between time periods, (45% vs. 47%, p = 0.50). Mortality was higher in the Post-2007 time period for CHD patients than non-CHD patients (11.1% vs. 2.4%, respectively; p < 0.001), while there was no difference noted during the Pre-2007 time period (6.5% vs. 6.6%, respectively; p = 0.95). Streptococcus was more common among CHD patients than non-CHD patients (27% vs. 17%), while Staphylococcus was more common among non-CHD patients than CHD patients (34% vs. 24%, p < 0.001). Even though the incidence of IE was stable over time, mortality was higher in CHD patients after the implementation of the 2007 AHA IE prophylaxis guidelines.
    Keywords:  Congenital heart disease; Infective endocarditis; National trends
    DOI:  https://doi.org/10.1007/s00246-018-2020-7
  9. JBI Database System Rev Implement Rep. 2018 Nov;16(11): 2224-2245
    Use of test weights for breastfeeding infants with congenital heart disease in a cardiac transitional care unit: a best practice implementation project.
    Courtney Gregory.
       OBJECTIVES: The goal of this project was to promote breastfeeding among infants with congenital heart disease in a quaternary care academic children's hospital. We aimed to increase the total number of breastfeeding episodes among all patients in the Pediatric Cardiac Transitional Care Unit with implementation of pre- and post-breastfeeding (test) weights.
    INTRODUCTION: Infants with congenital heart disease are able to breastfeed, but are often not encouraged to do so by healthcare providers. Fears and concerns relating to the inability to account for exact intake volumes through breastfeeding often prevent providers from supporting breastfeeding in these patients.
    METHODS: This project used Joanna Briggs Institute's Practical Application of Clinical Evidence System (JBI PACES) and Getting Research into Practice (GRiP) audit tool for promoting health practice change. A baseline medical record audit was conducted, followed by staff nurse education on breastfeeding test weights and the development of a parent education program. One follow-up audit measured compliance with best practice criteria for using breastfeeding test weights in infants with congenital heart disease.
    RESULTS: Compliance rates for use of breastfeeding test weights and provision of parent education, with baseline rates of 7% and 0%, respectively, rose to 90%. Identification of maternal intent to breastfeed upon admission to the unit increased to 78% compliance from 0%. Eighty-four percent of healthcare staff were educated on breastfeeding test weights.
    CONCLUSIONS: The safety of breastfeeding very ill infants was established. Breastfeeding episodes of all patients in the unit significantly increased after implementation of breastfeeding test weights and mothers of the sample patients expressed more confidence in breastfeeding their sick infants. Although barriers to breastfeeding for patients with congenital heart disease still persisted, this project had a positive impact on the culture of breastfeeding within the project unit. Increased healthcare provider education on breastfeeding will be essential to sustaining outcomes of this implementation.
    DOI:  https://doi.org/10.11124/JBISRIR-2017-003759
  10. Asian Cardiovasc Thorac Ann. 2018 Nov 11. 218492318814079
    Analysis of achieving an "ideal" outcome following midline unifocalization.
    Richard D Mainwaring, William L Patrick, Tatiana R Rosenblatt, Teimour Nasirov, Komal Kamra, Frank L Hanley.
       OBJECTIVE: Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is a complex form of congenital heart disease. Midline unifocalization has been developed for the surgical treatment of this condition. There are 3 outcome measures that determine long-term success: patients are alive, patients have achieved complete repair (i.e. ventricular septal defect closure), and patients have a relatively low right ventricle-to-aortic pressure ratio (<0.45). However, to date, no studies have combined these 3 outcome measures to analyze the likelihood of achieving an ideal outcome.
    METHODS: This was a retrospective review of 255 patients who underwent midline unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. The median age at unifocalization was 4.5 months.
    RESULTS: Two hundred thirty-five (92%) patients were alive at a mean follow-up of 5.3 years. Two hundred and seventeen (85%) patients underwent single-stage complete repair, and 38 (15%) had an initial unifocalization and shunt. Twenty-four of the 38 palliated patients have subsequently undergone repair. Thus 241 (94%) patients ultimately achieved complete repair. Of the 241 patients who were repaired, 219 (86%) had a right ventricle-to-aortic peak systolic pressure ratio <0.45. Combining these outcome measures, 77% of patients achieved an ideal outcome at one month, 73% at 6 months, 76% at one year, and 80% at 4 years.
    CONCLUSIONS: Most (80%) patients can achieve all 3 measures of favorable outcome at 4 years following midline unifocalization. We speculate that this will be a favorable portend for the future of these patients.
    Keywords:  Abnormalities; Heart disease; Heart septal defects; Pulmonary atresia; Reconstructive surgical procedures; Treatment outcome; congenital; multiple; ventricular
    DOI:  https://doi.org/10.1177/0218492318814079
  11. Int J Cardiol. 2018 Oct 27. pii: S0167-5273(18)33643-X. [Epub ahead of print]
    Resuscitation for out-of-hospital cardiac arrest in adults with congenital heart disease.
    Jim T Vehmeijer, Michiel Hulleman, Joey M Kuijpers, Marieke T Blom, Hanno L Tan, Barbara J M Mulder, Joris R de Groot, Rudolph W Koster.
       AIMS: Adult congenital heart disease (ACHD) patients are at increased risk of sudden cardiac death and out-of-hospital cardiac arrest (OHCA). Currently, insufficient data exist on outcome, causes and circumstances of OHCA of ACHD patients resuscitated for OHCA. We investigate these parameters in ACHD patients in comparison to OHCA in the general population.
    METHODS AND RESULTS: We identified ACHD patients with OHCA by linking data from a Dutch nationwide registry of ACHD patients (CONCOR, n = 15,727), and ARREST, a cohort of OHCA cases (n = 17,868). 62 ACHD patients with OHCA were identified. Ventricular septal defect (n = 11), bicuspid aortic valve (n = 10) and atrial septal defect (n = 8) were the most common diagnoses. We included OHCA cases from the general population as controls. ACHD patients were younger than controls (n = 11,624) at the time of OHCA (47 (SD ± 17) years vs. 66 (SD ± 15) years, respectively, p < 0.001), and more often had a shockable initial rhythm (67% vs 40%, respectively, p < 0.001). A cardiac cause of OHCA was identified in 76% of ACHD patients, with only 7% due to myocardial infarction or ischemia. Survival was better in ACHD patients than in controls (44% vs. 19%, p < 0.001), but this difference disappeared after correction for age, gender, witnessed arrest, bystander resuscitation, public location and shockable rhythm.
    CONCLUSIONS: OHCA in ACHD patients occurs at young age, is rarely caused by ischemia and occurs mainly in patients with simple congenital defects. Risk stratification efforts should therefore not be restricted to ACHD patients with severe congenital defects.
    Keywords:  Cardiopulmonary resuscitation; Congenital heart disease; Defibrillation; Out-of-hospital cardiac arrest; Sudden cardiac death
    DOI:  https://doi.org/10.1016/j.ijcard.2018.10.096
  12. Curr Opin Cardiol. 2018 Nov 15.
    Long-lasting benefits of exercise for those living with a Fontan circulation.
    Rachael Cordina, Yves d'Udekem.
       PURPOSE OF REVIEW: In recent decades, the physiology and late outcomes of the Fontan circulation have become better characterized and interest has turned to interventions that might improve exercise capacity and clinical sequelae. No therapy has currently been shown to be more effective for improving exercise capacity in the setting of a Fontan circulation than exercise. This review examines the current evidence supporting exercise as therapy for this group.
    RECENT FINDINGS: People living with a Fontan circulation are at increased risk for reduced skeletal muscle mass and exercise capacity with important implications for clinical outcomes. Small studies suggest that performing moderate-to-vigorous aerobic and resistance exercise improves exercise capacity through unique mechanisms such as superior respiratory and skeletal muscle function and venous return.
    SUMMARY: Despite traditional concerns about moderate-to-vigorous physical activity, current evidence suggests that exercise is beneficial for the Fontan circulation and that, after appropriate screening, we should be encouraging regular moderate-to-vigorous physical activity. Multicentre randomized controlled trials are needed that further explore mechanisms, late impact for end-organ function and strategies to optimize long-term moderate-to-vigorous physical activity levels.
    DOI:  https://doi.org/10.1097/HCO.0000000000000582
  13. Curr Treat Options Cardiovasc Med. 2018 Nov 12. 20(12): 102
    State of the Art Management of Mechanical Heart Valves During Pregnancy.
    Shivani R Aggarwal, Katherine E Economy, Anne M Valente.
       PURPOSE OF THE REVIEW: To review the management of women with mechanical heart valves during pregnancy, from preconception counseling through delivery with a summary of the latest guidelines.
    RECENT FINDINGS: The hypercoagulability of pregnancy combined with the imperfect choices of anticoagulant agents contribute to a high risk of complications in pregnant women with mechanical heart valves. Valve thrombosis remains a major concern, much of which occurs during the first trimester transition to heparin-based products. The safest method of anticoagulation, with the best balance of maternal and fetal risk, is use of low-dose vitamin K antagonists, but only if therapeutic anticoagulation can be achieved with warfarin doses of ≤ 5 mg/day. Management of mechanical heart valves in pregnancy remains fraught with difficult decisions involving balancing of maternal and fetal risks as well as a high risk of maternal and fetal complications. Preconception counseling and planning is imperative. A risk-benefit discussion with the patient will help guide the choice of anticoagulation and outline the plan for safe delivery options. A multidisciplinary approach to management is advisable with close follow-up and care in a tertiary center.
    Keywords:  Congenital heart disease; Mechanical heart valves; Pregnancy; Valvular heart disease
    DOI:  https://doi.org/10.1007/s11936-018-0702-3
  14. Crit Care Med. 2018 Nov 12.
    Extracorporeal Cardiopulmonary Resuscitation: One-Year Survival and Neurobehavioral Outcome Among Infants and Children With In-Hospital Cardiac Arrest.
    Therapeutic Hypothermia After Pediatric Cardiac Arrest (THAPCA) Trial Investigators
       OBJECTIVE: To describe neurobehavioral outcomes and investigate factors associated with survival and survival with good neurobehavioral outcome 1 year after in-hospital cardiac arrest for children who received extracorporeal cardiopulmonary resuscitation.
    DESIGN: Secondary analysis of the Therapeutic Hypothermia after Pediatric Cardiac Arrest In-Hospital trial.
    SETTING: Thirty-seven PICUs in the United States, Canada, and the United Kingdom.
    PATIENTS: Children (n = 147) resuscitated with extracorporeal cardiopulmonary resuscitation following in-hospital cardiac arrest.
    INTERVENTIONS: Neurobehavioral status was assessed using the Vineland Adaptive Behavior Scales, Second Edition, at prearrest baseline and 12 months postarrest. Norms for Vineland Adaptive Behavior Scales, Second Edition, are 100 (mean) ± 15 (SD). Higher scores indicate better functioning. Outcomes included 12-month survival, 12-month survival with Vineland Adaptive Behavior Scales, Second Edition, decreased by less than or equal to 15 points from baseline, and 12-month survival with Vineland Adaptive Behavior Scales, Second Edition, greater than or equal to 70.
    MEASUREMENTS AND MAIN RESULTS: Of 147 children receiving extracorporeal cardiopulmonary resuscitation, 125 (85.0%) had a preexisting cardiac condition, 75 (51.0%) were postcardiac surgery, and 84 (57.1%) were less than 1 year old. Duration of chest compressions was greater than 30 minutes for 114 (77.5%). Sixty-one (41.5%) survived to 12 months, 32 (22.1%) survived to 12 months with Vineland Adaptive Behavior Scales, Second Edition, decreased by less than or equal to 15 points from baseline, and 39 (30.5%) survived to 12 months with Vineland Adaptive Behavior Scales, Second Edition, greater than or equal to 70. On multivariable analyses, open-chest cardiac massage was independently associated with greater 12-month survival with Vineland Adaptive Behavior Scales, Second Edition, decreased by less than or equal to 15 points and greater 12-month survival with Vineland Adaptive Behavior Scales, Second Edition, greater than or equal to 70. Higher minimum postarrest lactate and preexisting gastrointestinal conditions were independently associated with lower 12-month survival with Vineland Adaptive Behavior Scales, Second Edition, decreased by less than or equal to 15 points and lower 12-month survival with Vineland Adaptive Behavior Scales, Second Edition, greater than or equal to 70.
    CONCLUSIONS: About one third of children survived with good neurobehavioral outcome 1 year after receiving extracorporeal cardiopulmonary resuscitation for in-hospital arrest. Open-chest cardiac massage and minimum postarrest lactate were associated with survival with good neurobehavioral outcome at 1 year.
    DOI:  https://doi.org/10.1097/CCM.0000000000003545
  15. Pediatr Cardiol. 2018 Nov 14.
    A Novel Brain Injury Biomarker Correlates with Cyanosis in Infants with Congenital Heart Disease.
    Lindsey McPhillips, Dipak Kholwadwala, Cristina P Sison, Dorota Gruber, Kaie Ojamaa.
      Cyanotic heart lesions are a complex subset of congenital heart disease (CHD) in which patients are desaturated until surgical repair or palliation. We hypothesized that a direct relationship would exist between degree of desaturation and presence of systemic inflammation and brain injury in unrepaired patients less than 1 year of age. The pre-operative desaturation with augmented systemic inflammation would predict a more complex post-operative course. Fifty patients with CHD were enrolled in this study and classified as cyanotic (O2 ≤ 90%) or acyanotic (O2 > 90%) based on SpO2. Serum inflammatory mediators measured included interleukins (IL)-6, IL-8, IL-12p70, IL-10, IL-1β, tumor necrosis factor (TNF)-α, interferon (INF)-γ; macrophage inhibitory factor (MIF) and a novel brain biomarker, phosphorylated neurofilament heavy subunit (pNF-H). Twenty-two cyanotic and 28 acyanotic subjects were enrolled with SpO2 of 78 ± 18% and 98 ± 2% (p < 0.001), respectively, and mean age of 72 days (range 2-303) and 102 days (range 1-274), respectively. Cyanotic vs acyanotic subjects had elevated serum IL-6 (6.6 ± 7.6 vs 2.9 ± 2.9 pg/ml, p = 0.019) and pNF-H (222 ± 637 vs 57 ± 121 pg/ml, p = 0.046), and both biomarkers correlated with degree of desaturation (Spearman rank-order correlation ρ = - 0.30, p = 0.037 and ρ = - 0.29 p = 0.049, respectively). Post-operative inotrope scores at 24 h and duration of mechanical ventilation correlated inversely with pre-operative oxygen saturation (ρ = - 0.380, p = 0.014 and ρ = - 0.362, p = 0.020, respectively). The degree of pre-operative desaturation correlated with a more complicated post-operative course supporting the need for advanced peri-operative therapy in this population.
    Keywords:  Brain injury; Bypass surgery; Congenital heart disease; Cytokines; Neurofilament; Systemic inflammation
    DOI:  https://doi.org/10.1007/s00246-018-2023-4
  16. Case Rep Womens Health. 2018 Oct;20 e00085
    Twin pregnancy complicated by total placenta previa in a Fontan-palliated patient: A case report.
    Aoi Morita, Saki Kido, Masahiro Hachisuga, Hazumu Nagata, Nobuhiro Hidaka, Kiyoko Kato.
      We present a case of a twin pregnancy in a Fontan-palliated woman that was complicated by total placenta previa. The patient was diagnosed with tricuspid atresia type II, and underwent the Fontan operation at 11 years of age. At 32 years of age, she was shown to have a dichorionic diamniotic twin pregnancy. A placenta previa was also noted. At 26 weeks' gestation, she had difficulty breathing, cardiomegaly, and worsening mitral regurgitation. At 29 weeks' gestation, an emergency cesarean section was performed, as the patient had massive genital bleeding. A postoperative cardiac catheterization demonstrated a leak from the lateral tunnel to the atrium, which was considered a cause of hypoxemia during the peripartum period. The cardiac workload in a twin pregnancy is greater, which places a Fontan-palliated patient at increased risk. Careful follow-up monitoring with multidisciplinary expertise is recommended.
    Keywords:  Fontan circulation; Placenta previa.; Twin pregnancy
    DOI:  https://doi.org/10.1016/j.crwh.2018.e00085
  17. Ann Thorac Surg. 2018 Nov 10. pii: S0003-4975(18)31630-8. [Epub ahead of print]
    Perioperative Exposure to Suspect Neurotoxicants from Medical Devices in Newborns with CHD (Commentary).
    Jennifer C Romano.
      
    DOI:  https://doi.org/10.1016/j.athoracsur.2018.09.060
  18. Eur J Prev Cardiol. 2018 Nov 14. 2047487318812505
    Strength training in congenital heart disease: A way to boost respiratory function?
    Dimitrios Ntelios, George Giannakoulas, Konstantinos Dimopoulos.
      
    DOI:  https://doi.org/10.1177/2047487318812505
  19. Pediatr Neurol. 2018 Oct 17. pii: S0887-8994(18)30298-4. [Epub ahead of print]
    Pediatric Infective Endocarditis and Stroke:A 13-Year Single-Center Review.
    Gui-Fang Cao, Qi Bi.
       OBJECTIVE: We explored the relationship between pediatric infective endocarditis and stroke.
    PATIENTS AND METHODS: All children encountered with infective endocarditis from January 2002 to December 2015 were included as our sample, and their medical records were comprehensively reviewed.
    RESULTS: Sixty children with infective endocarditis were identified, including 30 boys and 30 girls aged eight months to 18 years (mean ± SD: 10.3 ± 5.6), and om 43 (71.6%) of these individuals had congenital heart disease. Left-sided endocarditis occurred in 25 patients (41.7%), and vegetations were found in 58 individuals (96.6%). The most often encountered microorganisms were Streptococcus viridans and Staphylococcus aureus, which were identified in five and four patients, respectively. Postendocarditis stroke occurred in nine patients, including five with cerebral infarction, two with intracerebral hemorrhage, and one with subarachnoid hemorrhage. The remaining child experienced cerebral infarction, intracerebral hemorrhage and subarachnoid hemorrhage simultaneously. The incidence of stroke in children with left-sided endocarditis was significantly higher than that of which in those who had right-sided endocarditis (32% versus 2.8%, P < 0.01). The most common manifestation of stroke was hemiparesis (55.5%). Two girls died of stroke, and the mortality rate in the patients who had stroke was significantly higher than that in those without stroke (22.2 % versus 3.9 %, P < 0.05).
    CONCLUSIONS: Our data indicate that stroke is common among children with infective endocarditis, especially in those with left-sided endocarditis, and major stroke may increase their risk of death. Congenital heart disease is the main underlying disease in children with infective endocarditis in China.
    Keywords:  Chinese children; cerebral embolism; congenital heart diseases; infective endocarditis; stroke
    DOI:  https://doi.org/10.1016/j.pediatrneurol.2018.07.001
  20. Ann Thorac Surg. 2018 Nov 10. pii: S0003-4975(18)31632-1. [Epub ahead of print]
    Hospital Distribution and Patient Travel Patterns for Congenital Heart Surgery in the United States (Commentary).
    James M Hammel.
      
    DOI:  https://doi.org/10.1016/j.athoracsur.2018.11.002
  21. BMC Med. 2018 Nov 12. 16(1): 205
    Selective serotonin reuptake inhibitor use during early pregnancy and congenital malformations: a systematic review and meta-analysis of cohort studies of more than 9 million births.
    Shan-Yan Gao, Qi-Jun Wu, Ce Sun, Tie-Ning Zhang, Zi-Qi Shen, Cai-Xia Liu, Ting-Ting Gong, Xin Xu, Chao Ji, Dong-Hui Huang, Qing Chang, Yu-Hong Zhao.
       BACKGROUND: In 2005, the FDA cautioned that exposure to paroxetine, a selective serotonin reuptake inhibitor (SSRI), during the first trimester of pregnancy may increase the risk of cardiac malformations. Since then, the association between maternal use of SSRIs during pregnancy and congenital malformations in infants has been the subject of much discussion and controversy. The aim of this study is to systematically review the associations between SSRIs use during early pregnancy and the risk of congenital malformations, with particular attention to the potential confounding by indication.
    METHODS: The study protocol was registered with PROSPERO (CRD42018088358). Cohort studies on congenital malformations in infants born to mothers with first-trimester exposure to SSRIs were identified via PubMed, Embase, Web of Science, and the Cochrane Library databases through 17 January 2018. Random-effects models were used to calculate summary relative risks (RRs).
    RESULTS: Twenty-nine cohort studies including 9,085,954 births were identified. Overall, use of SSRIs was associated with an increased risk of overall major congenital anomalies (MCAs, RR 1.11, 95% CI 1.03 to 1.19) and congenital heart defects (CHD, RR 1.24, 95% CI 1.11 to 1.37). No significantly increased risk was observed when restricted to women with a psychiatric diagnosis (MCAs, RR 1.04, 95% CI 0.95 to 1.13; CHD, RR 1.06, 95% CI 0.90 to 1.26). Similar significant associations were observed using maternal citalopram exposure (MCAs, RR 1.20, 95% CI 1.09 to 1.31; CHD, RR 1.24, 95% CI 1.02 to 1.51), fluoxetine (MCAs, RR 1.17, 95% CI 1.07 to 1.28; CHD, 1.30, 95% CI 1.12 to 1.53), and paroxetine (MCAs, RR 1.18, 95% CI 1.05 to 1.32; CHD, RR 1.17, 95% CI 0.97 to 1.41) and analyses restricted to using women with a psychiatric diagnosis were not statistically significant. Sertraline was associated with septal defects (RR 2.69, 95% CI 1.76 to 4.10), atrial septal defects (RR 2.07, 95% CI 1.26 to 3.39), and respiratory system defects (RR 2.65, 95% CI 1.32 to 5.32).
    CONCLUSIONS: The evidence suggests a generally small risk of congenital malformations and argues against a substantial teratogenic effect of SSRIs. Caution is advisable in making decisions about whether to continue or stop treatment with SSRIs during pregnancy.
    Keywords:  Antidepressant; Cohort studies; Congenital malformations; Meta-analysis; Pregnancy; Serotonin uptake inhibitors
    DOI:  https://doi.org/10.1186/s12916-018-1193-5
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